Aminoacyl-tRNA synthetases are a class of enzymes that charge tRNAs with their cognate amino acids. The protein encoded by this gene is an enzyme belonging to the class II family of aminoacyl-tRNA synthetases. Functioning in the synthesis of histidyl-transfer RNA, the enzyme plays an accessory role in the regulation of protein biosynthesis. The gene is located in a head-to-head orientation with HARS on chromosome five, where the homologous genes likely share a bidirectional promoter. Mutations in this gene are associated with the pathogenesis of Perrault syndrome, which involves ovarian dysgenesis and sensorineural hearing loss. Alternative splicing results in multiple transcript variants of this gene.
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Protein Aliases: EC 184.108.40.206; HARS-related; HisRS; histidine translase; histidine tRNA ligase 2, mitochondrial (putative); Histidine--tRNA ligase, mitochondrial; Histidine--tRNA ligase-like; histidine-tRNA ligase homolog; Histidyl-tRNA synthetase; histidyl-tRNA synthetase 2; histidyl-tRNA synthetase 2, mitochondrial (putative); probable histidyl-tRNA synthetase, mitochondrial
Gene Aliases: HARS2; HARSL; HARSR; HO3; PRLTS2
UniProt ID: (Human) P49590
Entrez Gene ID: (Human) 23438
Molecular Function: aminoacyl-tRNA synthetase